A 68-year-old patient visited our hospital, because of progressive renal failure. Laboratory data were serum creatinine 13.3mg/dl. BUN 74.3mg/dl, AGBMA 134U, MPO-ANCA 37% and CIC 1.6g/dl. Hemodialysis was performed on admission and renal biopsy was conducted. Though the histological findings showed fibrocelluler crescents and global sclerosis in many glomeruli, renal function was gradually ameliorated by administration of prednisolone (40 mg/day) and cyclophosphamide (50mg/day). After 3 weeks of therapy, hemodialysis was withdrawn temporarily. Because of the complication of pneumocystis carini, prednisoloned cyclophosphamide were discontinued, and maintenance hemodialysis was resumed. In spite of progressed histological findings, hemodialysis was withdrawn at least temporarily through the use of pharmacological therapy. Thus, the presence of ANCA may indicate a relatively good prognosis of AGBMA nephritis.